POS0710 INTERSTITIAL LUNG DISEASE IN PRIMARY SJÖGREN’S SYNDROME: CLINICAL AND LABORATORY FEATURES, EFFECTIVENESS OF RITUXIMAB THERAPY.
Authors: B. Chalcev, A. Torgashina, E. Sokol, J. Khvan
Journal: Annals of the Rheumatic Diseases (2022)

Background Interstitial lung disease (ILD) often occurs in primary Sjogren’s syndrome (pSS) and reduces the survival of patients [1, 2]. Diagnosis and therapy in pSS-ILD are not standardized. Objectives To evaluate clinical characteristics of pSS-ILD and efficacy of rituximab therapy in this group of patients. Methods We conducted retrospective study which included 19 pSS-ILD patients. pSS was diagnosed based on the ACR 2012 or ACR/EULAR 2016 criteria. Diagnosis and dynamic assessment of ILD was carried out on the basis of high-resolution computed tomography (HRCT) and pulmonary function tests (PFT). The median age of pSS debut was 49 years (7-72 years), the median age of ILD debut – 57 years (29-75 years). All 19 patients received rituximab: 10 as a monotherapy, 6 in combination with cyclophosphamide, 3 in combination with mycophenolate mofetil. The median follow-up duration was 24 months (3-108 months). Results In 5 of 19 patients (26%), ILD was the first manifestation of pSS, while 14 patients (74%) developed ILD after an average of 9 years of disease course in the absence of therapy or taking low doses of glucocorticoids and hydroxychloroquine. The most common patterns of ILD were nonspecific interstitial pneumonia (52,6%) and lymphocytic interstitial pneumonia (26,3%), while organizing pneumonia and usual interstitial pneumonia were significantly less common (10,5% respectively). Both patients with organizing pneumonia were diagnosed with pulmonary lymphoma (also they had persistent salivary gland enlargement, lymphadenopathy and paraproteinemia). When analyzing clinical and laboratory features, we did not identify any predictors of ILD in pSS (Table 1). Rituximab therapy was effective in 16 of 19 patients (84,2%) based on stabilization or improvement in HRCT and PFT. The therapy was ineffective in 3 patients (15,8%), one of whom (patient with pulmonary lymphoma) died from an acute exacerbation of ILD. Table 1. Clinical and laboratory features of pSS-ILD patients (N=19). Sign Frequency, % Asymptomatic course of ILD 36,8 ANA+ 100 AntiRo+ 73,7 AntiLa+ 47,4 RF IgM + 55 High CRP 28 High ESR 33 Hypocomplementemia 37,5 Hypergammaglobulinemia 44 High IgG 39 mIg 36 Cryoglobulinemia 18 Severe xerostomia, xerophthalmia 44 Focus score&gt;4 44 Vasculitis 26 Lymphoma 31,6 Conclusion Considering asymptomatic cases and the absence of pSS-ILD predictors, all patients with pSS at the onset of the disease and in the dynamics should undergo HRCT and PFT for the purpose of ILD early diagnosis. In the present study, rituximab therapy was highly effective in pSS-ILD, further prospective studies are needed to confirm these data. In patients with pSS-ILD, vigilance for pulmonary lymphoma is necessary, especially in the presence of persistent salivary gland enlargement, lymphadenopathy, paraproteinemia, pattern of organizing pneumonia and a lack of response to rituximab and cyclophosphamide therapy. References [1]Ito I, Nagai S, Kitaichi M, et al. Pulmonary manifestations of primary Sjogren’s syndrome: a clinical, radiologic, and pathologic study. Am J Respir Crit Care Med. 2005;171(6):632-638. doi:10.1164/rccm.200403-417OC [2]Gao H, Zhang XW, He J, et al. Prevalence, risk factors, and prognosis of interstitial lung disease in a large cohort of Chinese primary Sjögren syndrome patients: A case-control study. Medicine (Baltimore). 2018;97(24):e11003. doi:10.1097/MD.0000000000011003 Disclosure of Interests None declared